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家族性自身免疫和家族性狼疮对阿联酋系统性红斑狼疮患者临床表现及疾病转归的影响。

The impact of familial autoimmunity and familial lupus on the clinical presentations and disease outcomes of SLE patients in the United Arab Emirates.

作者信息

Al Dhaheri Afra, Alblooshi Hiba, Bharathan Anjali, Alneyadi Asma, Al Ali Maryam, Alzaabi Amna, Trad Jalal, Chandrasekhar Nair Satish, Aljaberi Najla

机构信息

Rheumatology Department, Tawam Hospital, Al Ain, UAE.

Department of Genetics and Genomics, College of Medicine & Health Sciences, UAE University, Al Ain, UAE.

出版信息

BMC Rheumatol. 2025 Oct 14;9(1):118. doi: 10.1186/s41927-025-00574-z.

Abstract

OBJECTIVE

The presentation and outcomes of Systemic lupus erythematosus (SLE) are influenced by ethnicity and genetic background. The United Arab Emirates (UAE) is one of the leading countries of SLE per recent reports. In this study, we evaluated the effect of positive family history (FHx) of SLE and autoimmunity on clinical presentations and disease outcomes.

METHODS

A retrospective observational study of patients seen between 2011 till 2023 was conducted. Included patients were those fulfilling the 2019 EULAR/ACR classification criteria. Comparative analyses were conducted between those with familial history of autoimmunity and SLE and those without.

RESULTS

Out of 279 SLE patients, a total of 241 patients fulfilled the 2019 EULAR/ACR classification criteria and were included in the study. There was positive FHx of autoimmunity in 27% of the study population, and positive FHx of SLE (in first-degree relatives, "familial SLE") in 14.5% of the study population. Comparisons between positive and negative FHx of autoimmunity/SLE showed younger age at diagnosis in those with positive FHx of autoimmunity (p-value = < 0.001) and higher frequency of Raynaud's phenomonen (p-value = 0.022). Patients with familial SLE were also younger at diagnosis (p- value = 0.004) and had more mucocutaneous features (p-value = 0.042).

CONCLUSION

The percentage of familial SLE in our UAE study population is 14.5% which is higher than reported in non-Arab study populations. In our study population, patients with familial SLE and familial autoimmunity tend to present earlier, while patients with familial SLE tend to have more mucocutaneous features than those without familial SLE.

CLINICAL TRIAL NUMBER

Not applicable.

摘要

目的

系统性红斑狼疮(SLE)的临床表现和预后受种族和遗传背景影响。根据近期报告,阿拉伯联合酋长国(UAE)是SLE发病率较高的国家之一。在本研究中,我们评估了SLE和自身免疫性疾病的阳性家族史(FHx)对临床表现和疾病预后的影响。

方法

对2011年至2023年间就诊的患者进行回顾性观察研究。纳入的患者均符合2019年欧洲抗风湿病联盟(EULAR)/美国风湿病学会(ACR)分类标准。对有自身免疫性疾病和SLE家族史的患者与无家族史的患者进行比较分析。

结果

在279例SLE患者中,共有241例患者符合2019年EULAR/ACR分类标准并纳入研究。研究人群中27%有自身免疫性疾病的阳性家族史,14.5%有SLE的阳性家族史(一级亲属中有“家族性SLE”)。自身免疫性疾病/SLE阳性和阴性家族史之间的比较显示,有自身免疫性疾病阳性家族史的患者诊断时年龄较小(p值<0.001),雷诺现象的发生率较高(p值=0.022)。家族性SLE患者诊断时年龄也较小(p值=0.004),且有更多的黏膜皮肤表现(p值=0.042)。

结论

在我们的阿联酋研究人群中,家族性SLE的比例为14.5%,高于非阿拉伯研究人群中的报告比例。在我们的研究人群中,有家族性SLE和家族性自身免疫性疾病的患者往往发病较早,而有家族性SLE的患者比无家族性SLE的患者往往有更多的黏膜皮肤表现。

临床试验编号

不适用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa38/12522406/272f954d474c/41927_2025_574_Fig1_HTML.jpg

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